Intrahepatic cholangiocarcinoma (ICC) is a rare tumor, with an increasing incidence worldwide and an overall poor prognosis. Symptoms are usually nonspecific, contributing to an advanced tumor stage at diagnosis. The staging system for ICC has recently been updated and is based on number of lesions, vascular invasion, and lymph node involvement. Complete surgical resection to negative margins remains the only potentially curable treatment for ICC. Gemcitabine-based adjuvant therapy can be offered based on limited data from patients with unresectable ICC. Overall 5-year survivals after resection range from 17% to 44%, with median survivals of 19 to 43months. © 2014 Elsevier Inc.